Pulmonary Vein Isolation (PVI) with Pulsed Field Ablation (PFA) in a Patient with Immunoglobulin G4-Related Disease: A Case Report

Immunoglobulin G4-Related Disease (IgG4-RD) is a systemic autoimmune condition characterized by tissue infiltration by IgG4-positive plasma cells and multisystemic fibrosis that can affect various organs including the cardiovascular system. Development of vascular fibrosis and cardiovascular disorders can severely affect patient prognosis with fatal consequences. Atrial Fibrillation (AF) is a common cardiac arrhythmia that can lead to significant morbidity and mortality. The interaction between IgG4-RD and AF is unknown. The inflammatory processes may contribute to the development of atrial fibrosis provoking AF. Pulmonary vein isolation (PVI) is a well-established treatment for AF, but its safety and efficacy in patients with underlying IgG4-RD has not been described yet.

Here, we present a case of a 61-year-old male with IgG4-RD with cholangitis, chronic aortitis of the aorta ascendens and stenosis of the inferior vena cava, under established corticoid therapy. He presented with a highly symptomatic AF EHRA IV and episodes after electrical cardioversion under antiarrhythmic therapy. Echocardiogram showed normal left ventricular function with atrial enlargement (LAVI 32ml/min).  A CT scan suggested fibrosis of the inferior vena cava with a significant subhepatic stenosis, making a potential catheter passage more complicated. Corticosteroid therapy led initially to a reduction in AF episodes; however increasing frequency of episodes despite corticosteroids led the patient to prefer PVI. The patient preferred therefore, due to the highly symptomatic AF to undergo PVI despite the elevated vascular risk. Vena cava inferior could be passed smoothly with a long sheath and PVI with pulsed field ablation (PFA) was performed without occurrence of peri- or postprocedural complications. To detect atrial fibrosis, as a correlation of IgG4-RD, biatrial 3D-electroanatomical mapping was performed. Despite biatrial dilatation (RAVI 215ml, LAVI 190ml), no significant fibrosis was detected. He remained in sinus rhythm without any recurrence of AF in 1-and 3-months follow-up. 

This is the first reported case of AF ablation in a patient with IgG4-RD. The presence of inflammation in IgG4-RD may influence the underlying pathophysiology of AF in this patient eventually affecting the efficacy and durability of AF ablation. Moreover, IgG4-RD related vascular fibrosis, chronic corticoid therapy and inferior vena cava stenosis raised the complexity and complications risk of a potential invasive strategy in this case. Due to the highly symptomatic AF and the patient’s preference, we decided to perform PVI. We performed biatrial 3D-electroanatomical mapping to detect atrial fibrosis, whereas the absence of fibrosis in this case could be indicative of an effective control of the underlying disease under established corticoid therapy. While there is currently no data on the safety and efficacy of PVI in patients with IgG4-RD, this case report demonstrates that it could be a successful treatment strategy. It is important to note that this is a single case report and further cases are necessary to evaluate the durability of AF ablation and the relationship between IgG4-RD and AF.

This case report highlights the successful use of PVI with PFA in a patient with IgG4-RD with vascular pathologies. While more research is needed, PVI may be a safe and effective treatment option for selected patients with IgG4-RD and AF.